Large cell neuroendocrine carcinoma of the ampulla of Vater

BACKGROUND: Neuroendocrine tumors of the ampulla of Vater are extremely rare, and few cases of large cell neuroendocrine carcinoma (LCNEC) of the ampulla have been reported. METHODS: A 48-year-old male with obstructive jaundice was admitted to our hospital. On examination the patient was found to have a periampullary growth and subsequently underwent the Whipple's procedure. RESULTS: Histopathological examination and immunohistochemistry revealed features of LCNEC of the ampulla of Vater. The patient developed multiple liver metastases 6 months after Whipple's procedure. CONCLUSION: LCNEC of the ampulla of Vater is rare and highly aggressive, with a dismal prognosis.     

Pancreatic invasion is a prognostic indicator after radical resection for carcinoma of the ampulla of Vater

Sixty-three patients who had undergone pancreatoduodenectomy for carcinoma of the ampulla of Vater were analyzed with respect to tumor extent and prognosis. The postoperative mortality rate was 3% and overall survival rates 3 and 5 years after surgery were 55% and 46%, respectively. pTNM stage did not reflect prognosis after resection in patients at stages 2 and 3, while pancreatic invasion and regional lymph node metastasis clearly reflected prognosis after resection. Of the 26 patients who had no pancreatic invasion, regional lymph node metastasis was seen in only 19%, whereas of the 37 patients with pancreatic invasion, 62% exhibited lymph node metastasis. These factors were significantly correlated (P<0.001). Pancreatic invasion appeared to be an indirect indicator of regional lymph node metastasis. We conclude that, to improve prognosis for patients with pancreatic invasion, extended resection including extended lymphadenectomy, is a preferable additional procedure.     

Carcinoid of the ampulla of Vater

Endocrine neoplasms only rarely occur at the ampulla of Vater, comprising mostly carcinoids and malignant carcinoids, as well as few cases of poorly differentiated endocrine carcinomas (small cell carcinomas). Only 105 cases are reported in the literature, most as single case reports. For many years, the neoplasms of the disseminated neuroendocrine cell system of the gastrointestinal tract have been subsumed as 'carcinoids'. Instead, in the latest World Health Organization (WHO) classification published in 2000, it is recommended to distinguish between (i) well-differentiated endocrine tumors (carcinoids); (ii) well-differentiated endocrine carcinomas (malignant carcinoids); and (iii) poorly differentiated endocrine carcinomas (small cell carcinomas). Patients with carcinoid tumors of the ampulla of Vater are very often free of clinical and laboratory findings that belong to the carcinoid syndrome. Approximately 26% of all patients with carcinoid tumor reported in the literature had neurofibromatosis. Besides endoscopic retrograde cholangiopancreatography, endosonography, computed tomography or magnetic resonance imaging may complete the staging approach of this tumor. The Kausch-Whipple procedure or pylorus-preserving pancreaticoduodenectomy is considered the treatment of choice for ampullary, well-differentiated carcinoids >2.0 cm and for ampullary neuroendocrine carcinomas. However, it should be considered that long-term survival of patients with ampullary carcinoids is also reported after local tumor excision (5-year survival rate of 90%). The dilemma is that the differentiation of neuroendocrine tumors cannot be assessed intraoperatively in most cases. Therefore, considering that the 5-year survival rate in patients with neuroendocrine carcinomas of the ampulla of Vater is very low without radical resection, neuroendocrine tumors of the ampulla of Vater without definite histological differentiation should undergo extended surgery.     

胰腺腺泡细胞癌1例报告

1病例资料患者女性,38岁,因＂间断性上腹部胀痛20 d＂入本院治疗。既往体健。查体：腹平坦,腹壁未见异常,脐部生理凹陷,体毛发育正常,未见上腹部搏动;腹壁柔软,上腹部轻压痛,无反跳痛及肌紧张,肝脾肋缘下不能触及,胆囊不能触及,Murphy征阴性,Courvoisier征阴性;全腹叩诊呈鼓音,肝区叩击痛阴性,胆囊区叩击痛阴性,胃泡区叩诊呈鼓音,移动性浊音阴性,水坑征阴性;听诊肠鸣音约4次/min,未闻及杂音。     

Signet-ring cell carcinoma of ampulla of Vater： Contrast-enhanced ultrasound findings

Signet-ring cell carcinoma （SRCC） of ampulla of Vater is extremely uncommon, and less than 15 cases have been reported so far in literature. It mainly occurs in elderly people （median age 57 years）. We report a rare case of SRCC of the ampulla of Vater in a 38-year-old woman who presented with a small tumor at the Vater, discovered by the contrast-enhanced ultrasound （CEUS）. Histopathological examination showed prominent signet-ring features. We also describe the imaging features of SRCC of ampulla of Vater in CEUS.     

Large cystic acinar cell carcinoma of the pancreas

Acinar cell carcinoma of the pancreas is a rare neoplasm exhibiting pancreatic enzyme production by the neoplastic cells. It has a highly characteristic cellular arrangement reflecting its acinar derivation, and a definite diagnosis is made based on immunohistochemical and ultrastructural results. Such tumors are often large but rather well circumscribed. Some cases have a cystic appearance due to hemorrhage or necrosis, but a large cystic mass appearing as a pseudocyst is quite rare. We present a large cystic acinar cell carcinoma of the pancreas.     

Small-cell neuroendocrine carcinoma of the ampulla of Vater

We report a patient (an 80-year-old woman) with anemia and fecal occult blood, who had an emergency operation for carcinoma of the cecum (well-differentiated adenocarcinoma without local lymph node metastasis). Postoperative magnetic resonance cholangiopancreatography, cholangiography, and upper gastroduodenal endoscopy showed a tumor of the ampulla of Vater, and pylorus-preserving pancreatoduodenectomy was performed. Histology of the resected tumor was that of small-cell carcinoma, and immunohistochemistry showed positive staining for neuron-specific enolase, chromogranin A, and synaptophysin, confirming the neuroendocrine nature of the tumor. As the histology of the tumor was distinct from cecal carcinoma, and no tumors were found in other organs, the tumor was diagnosed as primary small-cell neuroendocrine carcinoma of the ampulla of Vater. The patient died due to multiple liver metastases of the carcinoma of the ampulla of Vater 7 months after the pancreatoduodenectomy. The clinical and morphological features of this disease have been reported in nine individuals previously.     

Usefulness of follow-up after pancreatoduodenectomy for carcinoma of the ampulla of Vater

Background: The prognosis for carcinoma of the ampulla of Vater (CAV) is better than for pancreatic cancer. The 5-year survival median rate after resection of CAV is 45%, but late recurrences remain possible. Several survival factors have been identified (lymph nodes, perineural invasion), but few data are available on the type of recurrences, their impact and their management. Patients and methods: A total of 41 patients treated by pancreatoduodenectomy (PD) for CAV from 1980 to 2003 were studied retrospectively. Patient selection, long-term survival recurrence rate and recurrence treatment were reviewed. Univariate and multivariate proportional hazards analysis were conducted on this series. Results: The mean follow-up was 48 months. Five-year survival was 62.8%. Eleven patients had recurrences (6–67 months). Recurrence was associated with time to all-causes death (hazard ratio [HR] 4.3, p=0.003). Factors predictive of recurrence were perineural invasion (HR 5.3, p=0.02), lymph node invasion (HR 5.3, p=0.02) and differentiation (HR 0.2, p=0.05). Three patients underwent surgical R0 treatment of their recurrences. Two who presented with solitary liver metastasis are alive and disease-free. Conclusions: Recurrence represents a serious threat in the prognosis of CAV after surgery. Some of these recurrences, in particular liver metastases, are accessible for a curative treatment. This finding supports the usefulness of a close and long-term follow-up after surgery to improve survival of patients with CAV, especially in the group of patients with a good prognosis.     

Pancreatic acinar cell carcinoma presenting as acute pancreatitis

BackgroundAcinar cell carcinoma (ACC) is a rare pancreatic neoplasm, and its presentation with acute pancreatitis has not been reported previously.Case outlineA 70-year-old man presented with acute pancreatitis, and a spiral CT scan showed a 5-cm tumour in the body of the pancreas. Distal pancreatectomy was performed, and histological examination showed an ACC.DiscussionThis is a newly reported mode of presentation for a rare pancreatic tumour     

Staging of carcinoma of the pancreas and ampulla of Vater

Summary Between 1984 and 1987, 472 Norwegian patients with histologically or cytologically verified carcinoma of the pancreas (N=442) and ampulla of Vater (N=30) were accrued and TNM staged according to UICC. The influence of the T, N, and M categories on long-term survival was evaluated. The T1a and T1b tumors of stage I pancreatic carcinoma had a comparable survival (p=0.68–0.95). A higher T category (T1–T3) predicted a more dismal prognosis (p=0.000). The T1 and T2 carcinomas of the ampulla of Vater had a comparable favorable prognosis, and the T3 and T4 tumors had a comparable unfavorable prognosis. The N1 vs N0 (p=0.000–0.01) and M1 vs M0 categories (p=0.00–0.003) predicted a more dismal prognosis for both pancreatic and ampullary carcinoma. By logistic regression analyses, pancreatic tumor extension into peripancreatic fat or nerves and invasion of ampullary carcinomas into duodenal wall, unfavorably influenced the N1 category (p=0.000–0.04) and tumor diameter influenced the M1 category (p=0.002–0.04) both for pancreatic and ampullary carcinoma. The T, N, and M categories all independently influenced survival of pancreatic carcinoma (p=0.000–0.003). Only the N category (p=0.01) influenced the prognosis of ampullary carcinomas.     

Coexistence of small cell neuroendocrine carcinoma and villous adenoma in the ampulla of Vater

Small cell neuroendocrine carcinoma of the ampulla of Vater is extremely rare and different from the common ampullary adenocarcinoma. The ampullary adenoma is also a rare neoplasm and has the potential to develop an adenocarcinoma. Their coexistence has been rarely reported in the literature. We herein describe an unusual case of a small cell neuroendocrine carcinoma associated with a villous adenoma in the ampulla of Vater with emphasis on computed tomography （CT） and histopathological findings. We also discuss their clinical, histopathological and radiological features as well as possible histogenesis.     

Small cell neuroendocrine carcinoma of the ampulla of Vater with foci of squamous differentiation: a case report

A 54-year-old woman with obstructive jaundice was found to have a 4-cm ulcerated, elevated tumor, located at the papilla of Vater by endoscopy and radiographic investigation. Based on a clinical diagnosis of carcinoma of the ampulla of Vater, a pylorus-preserving pancreaticoduodenectomy was performed. The histologic appearance of the lesion was identical to extrapulmonary small cell carcinoma, with diffuse proliferation of small, spindle-shaped, atypical tumor cells with numerous mitoses. Neuroendocrine differentiation was demonstrated by immunoreactivity with neuron-specific enolase (NSE) and Leu-7, and by the presence of dense core granules ultrastructurally. The tumor was composed mainly of small cell neuroendocrine carcinoma, and partially of an area of squamous differentiation, showing transition from one to the other. Histopathologic investigation disclosed direct invasion deep to the lamina propria, and infiltration of the pancreatic parenchyma, duodenum, and bile duct. Lymph node metastases were present in the superior pancreaticoduodenal chain. The patient died of liver metastases 8 months later. As in previously reported cases of small cell neuroendocrine carcinoma of the ampullary region, the present case showed extremely aggressive clinical behavior with early metastases resulting in fatal outcome.     

Feasibility of endoscopic papillectomy in early stage ampulla of Vater cancer

Background and Aim:  Although endoscopic papillectomy has been attempted in early stage ampullary cancer (pTis, T1), its curative role and indications remain uncertain. The present study was designed to assess the factors that predict malignancy and lymph node metastasis and to suggest potential indications for endoscopic papillectomy by analyzing clinicopathological data.
Methods:  We performed a retrospective analysis of clinical and histopathological data of 216 patients with ampullary cancer between 1991 and 2006.
Results:  No tumor in pTis stage had metastasized to lymph nodes and only 9% of tumors in pT1 had metastasized. Tumor size ( P  = 0.018), depth of invasion ( P  = 0.021) and venous invasion ( P  = 0.014) were found to be significantly related to lymph node metastasis. Cases with early stage ampullary cancer of less than 2 cm with a well-differentiated histology and no angiolymphatic invasion ( n  = 13) showed no lymph node metastasis and no recurrence during a median follow up of 35.9 months.
Conclusion:  Endoscopic papillectomy can be adopted as a viable alternative to surgery in patients with early stage ampullary cancer of less than 2 cm in size and with a well-differentiated histology. When a resected specimen has a well-differentiated histology, and there is no resection margin involvement and no angiolymphatic invasion, our findings indicate that subsequent radical surgery is unnecessary.     

KL-6 mucin expression in carcinoma of the ampulla of Vater： Association with cancer progression

AIM: To assess histochemical expression of KL-6 and its clinicopathological significance in carcinoma of the ampulla of Vater. METHODS: Ampullary carcinoma tissues were collected from 38 patients who underwent pancreatoduodenectomy or local resection. Tissues were subjected to immunohistochemical analysis using KL-6 antibody. RESULTS: Positive staining of ampullary carcinoma cells was observed in 26 (68.4%) cases. Staining was not found in the surrounding non-cancer regions of the ampullary tissues. Remarkable KL-6 expression was observed in invasive carcinoma cells in pancreatic and duodenal tissues and in metastatic carcinoma cells in lymph nodes. Positive KL-6 expression was related to lymph node metastasis (P=0.020), pancreatic invasion (P=0.016), duodenal invasion (P= 0.034), and advanced stage of TNM clinical classification (P=0.010). Survival analysis showed that positive expression of KL-6 was related to a poorer prognosis (P=0.029). CONCLUSION: The aberrant expression of KL-6 mucin is significantly related to unfavorable behaviors of carcinoma of the ampulla of Vater.     

内镜下十二指肠乳头切除术在乳头部肿瘤诊断中的价值

目的:评价内镜下十二指肠乳头切除术(endos-copic papillectomy,EP)在乳头部肿瘤诊断中的临床价值.方法:分析2008-01/2012-03来我院的16例经病理检查确诊的十二指肠乳头部肿瘤患者的相关资料,评价EP在十二指肠乳头部肿瘤诊断中的临床价值.结果:16例患者中,内镜活检诊断为腺瘤者12例,慢性炎症改变者4例.EP将乳头部肿瘤组织切除后整体送检行病理学检查确诊为低分化腺癌者2例,高分化腺癌1例,腺瘤13例(其中1例腺瘤恶变,4例常规内镜活检诊断为慢性炎症改变者术后均确诊为腺瘤),EP术后准确率明显高于内镜活检法(P<0.05).超声内镜(endoscopic ultrasonography,EUS)检查提示病变均起源于黏膜层,胰胆管扩张者9例,未见黏膜下层连续性中断及胰胆管受累者,无腹膜后淋巴结肿大者;12例高回声病变者术后病理检查结果均为腺瘤,1例高回声病变局部见低回声区者术后病理检查结果为腺瘤恶变,3例低回声病变者术后病理检查结果均为腺癌.EP术后2例低分化腺癌者、1例腺瘤恶变者及1例腺瘤者胆管开口处见病变残留,提示胆管受累,但术前EUS检查均未提示胆管受累;1例高分化腺癌及11例腺瘤者病灶均完整切除,术后切缘阴性.EP术后除2例出现黑便外,无急性胰腺炎、穿孔及其他并发症发生,无患者死亡.结论:EP术将乳头部肿瘤组织切除后整体送检行病理学检查可提高诊断准确率,为治疗方法的抉择提供科学依据.     

Trends in incidence and management of cancer of the ampulla of Vater

AIM:To provide trends in incidence,management and survival of cancer of the ampulla of Vater in a welldefined French population.METHODS:Data were obtained from the populationbased digestive cancer registry of Burgundy over a34-year period.Age-standardized incidence rates were computed using the world standard population.Average annual variations in incidence rates were estimated using a poisson regression.A univariate and multivariate relative survival analysis was performed.RESULTS:Age-standardized incidence rates were0.46 and 0.30 per 100000 inhabitants for men and women,respectively.Incidence rate increased from0.26(1976-1984)to 0.58(2003-2009)for men and remained stable for women.Resection for cure was performed in 48.3%of cases.This proportion was stable over the study period.Among cases with curative resection,pancreatico-duodenectomy was performed in94.0%of cases and ampullectomy in 6.0%of cases.A total of 50.8%of cancers of the ampulla of Vater were diagnosed at an advanced stage.Their proportion remained stable throughout the study period.The overall1-and 5-year relative survival rates were 60.2%and27.7%,respectively.Relative survival did not vary over time.Treatment and stage at diagnosis were the most important determinants of survival.The 5-year relative survival rate was 41.5%after resection for cure,9.5%after palliative surgery and 6.7%after symptomatic treatment.In multivariate analysis,only stage at diagnosis significantly influenced the risk of death.CONCLUSION:Cancer of the ampulla of Vater is still uncommon,but its incidence increased for men in Burgundy.Diagnosis is often made at an advanced stage,dramatically worsening the prognosis.     

Large acinar cell carcinoma of the pancreas in a patient with elevated serum AFP level

A pancreatic carcinoma, associated with elevated serum alpha‐fetoprotein level, was resected from a 67‐year‐old man. The tumor was strongly suggested to be an acinar cell carcinoma of the pancreas, based on the histological findings of the resected specimen. The tumor measured 12 × 10 × 9 cm, and the cut surface was soft, whitish‐yellow, focally necrotic, and hemorrhagic. Under a light microscope, the tumor cells were not arranged in a tubular and trabecular pattern, but rather, showed a tendency toward an acinar structure. Immunohistochemically, α 1‐antitrypsin‐ and α 1‐antichymotrypsin‐positive reactions were diffusely positive in most of the tumor cells, while staining for chromogranin, neuron‐specific enolase, Grimelius, glucagon, insulin, and alpha‐fetoprotein was negative in the tumor cells. We report a large acinar cell carcinoma (associated with elevated serum alpha‐fetoprotein level), which had been misdiagnosed as hepatocellular carcinoma preoperatively.     

Signet ring cell carcinoma of the ampulla of Vater

Introduction

Signet ring cell carcinoma of the ampulla of Vater is a rare entity and less than 20 cases have been described in the literature. We report the cases of two patients with this disease and provide a literature review of previous studies.

Case report

We describe two patients with obstructive jaundice. Abdominal ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic and common bile duct. Duodenoscopy indicated a protruding mass on the ampulla of Vater. Histopathological examination showed round cells and their nuclei were located on one side with prominent signet-ring features. One patient underwent a cephalic pancreatoduodenectomy with lymphadenectomy and the other a total pancreatectomy.

Discussion

Signet ring cell carcinoma of the ampulla of Vater has only been described in isolated cases in the literature. Therefore, the clinicopathological features and prognosis of this disease have not yet been well defined.     

Acinar cell carcinoma with hypervascularity

Acinar cell carcinoma is an uncommon malignancy with a reported incidence of 1% among exocrine tumors of the pancreas. The case of a 60-year-old Taiwanese man who presented with obstructive jaundice, abdominal pain, and body weight loss is described here. A mixed clinical picture of islet cell tumor and ductal carcinoma of the pancreas was shown to be a hypervascular tumor at the pancreatic head region with an irregular stricture at the common channel of the common bile and pancreatic ducts. The patient had normal levels of plasma carcinoembryonic antigen, carbohydrate antigen 19-9, alpha-fetoprotein, but an increase in plasma levels of insulin and C-peptide. Immunohistochemical stains and electron microscopic examination of the tumor was consistent with acinar cell carcinoma.